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Clinical Sessions.

Clinical sessions of the Society were held on Thursday morning, May 24th, at the Clinical Laboratory of the Yale Medical School, at the New Haven Hospital, at Grace Hospital and at the Hospital of St. Raphael. The complete programme of these sessions will be found in the report of the Committee on Scientific Work.

Requests for reports of the clinical meetings were made of all physicians who took part in them. The following have been received:

NEW HAVEN HOSPITAL.

Dr. Hartshorn showed patients demonstrating the results of operation in the following condition:

(1) Imperforate anus in a child with recto-vaginal fistula.

(2) Congenital harelip.

(3) Fracture of patella.

(4) Halsted-Andrews operation for hernia.

Dr. Mailhouse showed a case of pseudo-muscular hypertrophy with evidences of bony malformation and derangements in the functions of the endocrine glands.

Dr. Blumer showed a case of paramyoclonos multiplex of which the following is a report:

Report of a Case of Paramyoclonus Multiplex

(Myokimia).

GEORGE BLUMER, M.D.

Since 1881, when Friedrich described the first case, there have appeared from time to time reports of a condition which has been called by various names. Friedrich's designation was paramyoclonus multiplex, but others have designated cases as myoclonus spinalis multiplex, myoclonia, myokimia, polyclonia and myospasia. The various conditions which have been grouped under these various names have only one symptom in common, namely, peculiar, almost lightning-like contractions of voluntary

muscles or parts of voluntary muscles. It is, indeed, very doubtful whether the term disease should be applied to these cases, it would seem hardly logical to speak of the condition even as a symptom-complex for the muscle changes are the sole abnormality in most instances. The following history details the findings in a patient referred by Dr. Joseph P. Ryan of Hartford in whom the condition existed:

L. L., aged 27, a Pole, married, a polisher of silver and plated ware. He complained of muscular twitchings.

The illness was of sudden onset, and dated back seven weeks. The day after lifting a heavy box of coal, the patient noticed headache and a burning sensation at the base of the skull. He had hot sensations in the feet and cold sensations in the ends of the fingers. He complained that after taking hot food he felt very weak, and stated that he had been unable to work since the onset of the illness. One week after onset he noted twitching of the muscles all over the body but especially in the legs, accompanied by a sensation of blood running through the affected parts. He states that at the time he occasionally had difficulty in voiding urine and was also constipated. He had cramps in the abdomen on urination or defecation. He had pains in the legs and shoulders and to a lesser extent all over the body. There were some tingling sensations in the hands and feet. There was slight loss of weight. He was sleeping rather badly. No circulatory, renal or digestive symptoms.

The patient had whooping cough and scarlet fever as a child. He had been in the United States eight years and had not been sick in that time. He denied venereal infection. He only occasionally takes alcohol and then in the form of beer. He smokes about forty cigarettes a week. He has been a polisher of silver plated ware for several years; this does not subject him to metallic poisoning.

The family history was negative. The patient's parents were dead of unknown causes. He has one brother and one sister alive and well. He married at twenty-two, and has three healthy children. His wife has had no miscarriages.

Examination shows a very poorly nourished individual. The malar eminences are prominent. The eyes are sunken and the lids are dark. The skin is sallow. The patient is sweating profusely. The pupils react to light and accommodation. There is no nystagmus. No facial or ocular paralyses are present. The tongue is flabby and heavily coated in the center. The throat is congested and oedematous, but the tonsils are not enlarged. The teeth are in good condition except for a slight gingivitis. There is no lead line on the gums. The thyroid gland is normal in size. There are a few slightly enlarged cervical lymph nodes on each side. The chest is emaciated and the supraclavicular fossae are depressed. The percussion note is slightly impaired at each apex and the breathing is harsher than normal, but there are no rales even on coughing. The heart

sounds are rather distant but clear; there is no enlargement of the heart by percussion. The blood pressure is 130/84. The abdomen is soft and the liver can be palpated at the costal margin. The spleen is not palpable. The lower pole of the right kidney is easily palpable. There are no masses in the abdomen. No scars seen on the genitalia. The testes are normal. There is no oedema of the shins.

Almost all the muscles showed marked changes. The myotatic irritability is increased in all the limb, face and trunk muscles. All over the body there appear, at intervals of a few seconds, sudden sharp contractions of groups of muscle fibers. These are much coarser than the fibrillary tremors of muscle atrophy. They are symmetrical but not synchronous. They involve groups of muscle fibers but not whole muscles. They do not result in movements of a limb or even of the fingers or toes. The patient is conscious of them and describes his sensation as blood running through the affected part. The muscles of the limbs are most affected, especially the lower limbs, those of the face less affected, and the eye muscles are not affected at all. Voluntary movement of a limb temporarily suspends the contractions. It was not noted whether they ceased in sleep. The electrical reactions were normal. Exposure to cold, excitement and mechanical irritation of the skin increased the movements.

The examination of the nervous system was practically negative. No sensory disturbances to touch, pain or temperature were present. There were no paralyses. The reflexes were all lively, but no pathological reflexes were present. Coördination was normal. There were no psychic changes.

The Wassermann reaction on the blood and spinal fluid was negative. The spinal fluid was clear and not under pressure. The Nonne test was negative, no cells seen.

The patient passed 740 cubic centimeters of urine in 24 hours. It was amber, clear, acid, with a specific gravity of 1026, a very faint trace of albumen and no sugar. Microscopically there were a few pus cells, but no casts. The examination by Dr. Underhill showed no creatin, creatinin I.10 grams and nitrogen 11.06 grams. in the 24-hour specimen. The hydrogen ion concentration was 6. The patient did not stay for further observation.

An examination of the feces by Dr. Underhill showed that no lead was present.

The blood showed 85 per cent of hemoglobin with 11,800 leucocytes. A differential count showed polymorphonuclears 72 per cent, large mononuclears, 5 per cent and small mononuclears 23 per cent. There was no stippling of the red cells.

An X-ray examination of the chest by Dr. Honeij showed no tuberculosis.

The patient left the hospital unimproved. During his stay the temperature ranged between F. 98 and F. 99, his pulse between 98 and 113, and his respiration between 20 and 23.

In order to compare this case with those so far reported a brief history of the condition is appended.

The etiology is obscure. Three quarters of the patients are males. Emotion, particularly fright, trauma, and in our case physical strain, directly preceded the onset of the disease in some instances. Tuberculosis has been described in a few patients, but in view of the prevalence of the disease this is probably coincidence. Lead poisoning was present in one reported case. Thyroid and parathyroid insufficiency have both been suggested.

The pathology is obscure as there have been but few autopsies. Degenerative changes in the cells of the anterior gray horns have been described but competent authorities differ as to the interpretation of the findings. Hunt found muscle fiber hypertrophy in his case. Some have ascribed the disease to peripheral muscular changes and a few believe cerebral lesions can explain it, but the consensus of opinion is to the effect that it is probably in the true organic cases a disease of the anterior gray horns of the cord.

The symptoms vary in different cases and it seems clear that the condition may appear as a manifestation of either functional or organic disease of the nervous system or as an isolated phenomenon of obscure origin and for the present regarded as idiopathic.

Since Friedrich's description, which was based on a single case, various groups of patients have been recognized. We may roughly classify them as follows:

I. Cases of the Friedrich type.

2.

Cases associated with functional nervous diseases such as hysteria or psychasthenia.

3. Cases associated with epilepsy.

4. Cases with sensory phenomena.

5. Cases associated with definite infections such as poliomyelitis. 6. Cases associated with mental deterioration (dementia).

Dana places cases confused with tic convulsif and a choreic type as separate subheadings but this hardly seems necessary as we are here dealing with errors in diagnosis, the muscle movements both in tic convulsif and chorea being quite different from those occurring in true myokimia. It is true that so-called chorea electrica is probably the same condition as myoclonia.

Stripped of the manifestations which are possibly chargeable to accompanying but independent disease the essential phenomenon of myokimia is the occurrence of sudden clonic contractions in single muscles or in

groups of muscle fibers. The muscles of the limbs are mainly affected, those of the face and trunk less often, the ocular muscles seldom if at all. The contractions are bilateral in distribution, but the same muscle does not contract simultaneously on both sides except on rare occasions. Voluntary motion usually tends to lessen but may increase the contractions; emotion, exposure to cold, and skin stimulation tend to increase During sleep they usually, but not invariably, disappear. The muscles react normally to electricity. Aside from the muscle change there is little of moment abnormal. Motion, sensation, mentality and coördination are usually normal. The reflexes are often exaggerated, but not necessarily so, and there are no pathological reflexes.

Of the variants from Friedrich's original picture two deserve special mention, namely the form associated with epilepsy and the cases associated with sensory phenomena.

The form associated with epilepsy was first described by Unverricht in his classical work on myoclonia published in 1891. These cases show a tendency to occur as a familial disease and the myoclonia occurs in the intervals between attacks.

The patients showing sensory disturbances constitute, according to Bastianelli, a separate group. They were described by Biancone who gave this form of myoclonus the name myokimia. Our patient belongs with this group in whom there is a special tendency to involvement of the face muscles as well as paraesthesiae and sometimes paralgesiae.

The diagnosis of myoclonus is not difficult but it may, at times, be hard to differentiate the neurasthenic or hysterical cases from those on an organic basis. In cases such as ours, where only groups of muscle fibers not subject to the will are involved, a functional origin is hardly conceivable. Where whole muscles are involved the differentiation may be more difficult but functional neuroses, except tic, are likely to involve synergic groups of muscles and moreover other signs of hysteria or psychasthenia are present. The epileptic subtype is, of course, easy to separate, and the so-called chorea electrica is held by many to be the same disease under a different name.

The prognosis of myoclonus is generally bad so far as recovery is concerned. Occasional recoveries are on record, but even these are dubious as remissions and subsequent relapses may occur. Usually the disease progresses slowly and at times ends fatally without complications.

Treatment is most unsatisfactory. An exciting cause should be sought for and removed if possible. Hydrotherapy and electricity alone or in combination seem the most satisfactory treatments. Various sedatives have been advised but their efficacy is very doubtful.

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